PB2220: MORTALITY IN PATIENTS WITH HEMOGLOBINOPATHIES AND (SARS-COV-2) COVID-19 INFECTION: SAUDI ARABIA EXPERIENCE
Authors: H. Ezzat, O. Kashari, D. Alissa, R. Almasuood, A. Al-jedai
Journal: HemaSphere (2022)

Background: Hemoglobinopathies are a group on inherited red blood cell disorders that include Sickle Cell Disease (SCD) and Thalassemia. Prevalence of hemoglobinopathy disorders in Saudi Arabia is high. However, information on clinical manifestations and mortality among patients with SARS-CoV-2 (COVID-19) infection in SCD and thalassemia is scarce. Aims: To explore patients’ characteristics, associated comorbidities, clinical manifestations, received treatment, and the main causes of death among in-hospital patients with COVID-19 infection who have hemoglobinopathies in Saudi Arabia during 2021. Methods: The study is a retrospective review of the Ministry of Health (MOH) COVID- 19 mortality database. The analysis included all reported deaths with hemoglobinopathy disorders and positive COVID-19 infection. Results: The study included 35 patients, 19 (54.3%) were males. Their age ranged from 16 to 63 years (Mean±SD: 40.5±13.2 years). A total of 25 patients (71.4%) were with sickle cell anemia, 6 (17.1%) with sickle cell trait, while each of a-thalassemia trait, b-thalassemia trait, b-thalassemia major and sickle b-thalassemia affected only one patient (2.9% each). Almost three-fourths of patients (26, 74.3%) were admitted to the intensive care units. Exchange transfusion was done for one patient (2.9%), top up blood transfusion (RBCs) was done for 18 patients (51.4%), fresh frozen plasma infusion for 6 patients (17.1%), platelets transfusion for 5 patients (14.3%), cryoprecipitate infusion for 4 patients (11.4%) and intravenous immunoglobulin infusion for two patients (5.7%). Clinical manifestations for COVID-19 among hemoglobinopathy patients were manifested as agitation (34, 97.1%), chest pain (33, 94.3%), diarrhea (31, 88.6%), confusion (29, 82.9%), cough (20, 57.1% each), oxygen desaturation (20, 57.1% each), fever (17, 48.6%), dyspnea (9, 25.7%), headache (2, 5.7%), abdominal colic (2, 5.7%), or vomiting (2, 5.7%). The main associated comorbidities were hypertension (8, 22.9%), diabetes (7, 20%), or chronic kidney diseases (7, 20%). The main received medications were anticoagulants, such as Enoxaparin (22, 62.9%), Heparin (14, 40%), norepinephrine (16, 45.7%), epinephrine (9, 25.7%), inotropes (20, 57.1%), Vasopressin (6, 17.1%), antivirals, such as Favipiravir (15, 42.9%), immunosuppressives, e.g., Methylprednisolone (8, 22.9%), dexamethasone (7, 20%), Tocilizumab (5, 14.3%) and antibiotics, such as Azithromycin (16, 45.7%), Ceftriaxone (8, 22.9%), Vancomycin (5, 14.3%), and Tazocin (3, 8.6%). The main causes for death among patients reported to the mortality database with hemoglobinopathies were septic shock (12, 34.3%), acute respiratory distress syndrome (8, 22.9%), multiple organ failure (5, 14.2%), renal failure (5, 14.2%), acute chest syndrome (5, 14.3%), pulmonary embolism (3, 8.6%), vaso-occlusive crisis (3, 8.6%), or heart failure (2, 5.7%), or disseminated intravascular coagulation (1, 2.9%). Summary/Conclusion: The mortality rate among patients with hemoglobinopathies is approximately 0.38 percent of all deaths reported with COVID-19 to the database. The main clinical manifestations for COVID-19 among hemoglobinopathy patients were agitation, chest pain, diarrhea, confusion, cough, oxygen desaturation, fever, and dyspnea. Causes of death are variable. Most of deaths were indirectly related to COVID-19 infection. The main received medications are anticoagulants, inotropes, antivirals, and antibiotics. Causes of mortality were mainly septic shock, multiple organ failure, renal failure, or acute chest syndrome.